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Surgery in People With Mild Haemophilia
Emerito Carlos Rodriguez-Merchan1
1Department of Orthopaedic Surgery, Hospital Universitario La Paz-IdiPaz, Madrid, Spain.
Summary
Surgery in people with mild hemophilia (PMH) can lead to higher risks of bleeding and inhibitors. Desmopressin use is recommended when possible, with combined therapy for high-risk cases.
Area of Science:
- Medical research
- Hematology
- Surgical procedures
Background:
- People with mild hemophilia (PMH) may require surgical interventions.
- Understanding surgical risks and management in PMH is crucial.
Purpose of the Study:
- To review and analyze existing literature on surgical procedures in patients with mild hemophilia.
- To identify risks and optimal management strategies for surgery in PMH.
Main Methods:
- A comprehensive literature search was conducted in PubMed (MEDLINE).
- Keywords used were "mild haemophilia surgery".
- Articles were screened for relevance, with 64 publications analyzed.
Main Results:
- PMH underwent various surgeries (e.g., pseudotumor, urological, dental) with generally satisfactory outcomes.
- However, PMH face increased risks of postoperative bleeding, mortality, and repeat surgeries compared to severe/moderate hemophilia.
- Peri-operative management often involved supra-therapeutic Factor VIII (FVIII) dosing, linked to inhibitor development, especially with the Arg593Cys genotype.
Conclusions:
- Desmopressin should be prioritized for inhibitor risk reduction in PMH whenever feasible.
- For PMH with high-risk mutations undergoing major surgery, a combination of desmopressin and FVIII concentrates is advisable.
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